About Cystic Fibrosis

Cystic fibrosis (CF) is an inherited disease that affects the respiratory and digestive systems. CF makes children sick by disrupting the normal function of epithelial cells — cells that make up the sweat glands in the skin and that also line passageways in the lungs, liver, pancreas, and digestive and reproductive systems.

A defect in these cells causes problems with the balance of salt and water in the body, leading it to produce thick mucus, which clogs the lungs, setting the stage for infections and other breathing problems. In kids with CF, this mucus also can prevent the normal absorption of key nutrients and fat in the intestines, leading to poor digestion, slow growth, difficulty gaining weight, greasy bowel movements, and a decreased ability to fight infection.

At the time of diagnosis, the majority of patients have a condition called pancreatic insufficiency. This means that enzymes created in the pancreas that digest fat, starch, and protein aren't properly passed into the intestines. As a result, the body has problems absorbing the necessary amounts of several important nutrients.

By providing good overall nutrition along with extra fat and calories and prescribed enzymes, you can help your child with CF grow healthy and strong.

Your Child's Nutritional Needs

In general, kids with CF need more calories than other kids in their age group. The amount of additional calories they need will vary according to each child's lung function, activity level, and illness.

A child's calorie needs might be even higher during an illness (even a low-grade infection can greatly increase the calories required). A CF dietitian can help you determine how many calories your child needs each day, and track growth and weight gain over time in order to provide a good nutrition plan.

Kids with CF also have some specific nutritional needs:

Calcium. Patients with pancreatic insufficiency have difficulty absorbing calcium and are especially at risk for developing osteoporosis (weak, brittle bones), as are all kids with CF. Dairy products are good sources of calcium (and the full-fat varieties also are good sources of fat and calories). Many juices also are fortified with calcium.

Essential fatty acids. Patients with cystic fibrosis have altered levels of plasma fatty acids. Found in plant oils, safflower oil, and soybean oils, they help in the building of cell membranes and may play a role in lung function. It is suggested that CF patients try to incorporate more omega 3 fatty acids into their diet, which can be done with either food sources or supplements. Salmon, flax seeds, and walnuts are excellent food sources of omega 3 fatty acids. Before giving your child any over-the-counter supplements, discuss this with your CF dietitian or doctor.

Fat-soluble vitamins (vitamins A, D, E, and K). Important for immune function, growth, and healing, these vitamins are absorbed along with fat. And because most kids with CF have trouble digesting fat, they often have low levels of fat-soluble vitamins and need to take specialized prescription supplements.

Iron. Many children with CF are at risk for developing iron-deficiency anemia, which can cause fatigue and poor resistance to infection. Fortified cereal, meats, dried fruits, and dark green vegetables are good sources of iron.

Salt. Kids with CF lose a lot of salt in their sweat, especially during hot weather and when they exercise. So a CF dietitian may suggest adding salt to an infant's formula and giving an older child salty snacks. The dietitian may recommend sending sports drinks along when your child plays sports or has phys ed, and during hot weather.

Zinc. Important for growth, healing, and fighting infection, zinc is found in meats, liver, eggs, and seafood.

Meals at Home and Away

To meet your child's nutrition needs, you'll work with the CF dietitian to create meal plans that include plenty of snacks, especially for toddlers who eat smaller meals.

For a picky eater, pay attention to the foods your child especially likes and adapt the menu as these preferences change. Ask an older child with CF to try one new food a week. It's also important to model good behavior for kids of all ages. Eat a well-balanced diet with a variety of foods and make sure you try new things yourself.

To make meals a pleasant experience:

Try to keep meals to about 20-30 minutes for toddlers and young kids, who can't sit still for extended periods.

Eat in a comfortable environment with few distractions.

Don't give huge portions that might seem overwhelming.

Offer praise for eating well, but don't force plates of food to be finished; encourage kids to eat as much as they can comfortably.

Keep food choices simple, especially for younger kids.

Make foods as attractive and appealing as possible.

All childcare providers and teachers should know that your child has CF and be aware of his or her nutritional and caloric needs. Work with the staff to plan high-calorie meals and snacks or send food if the menu can't accommodate your child's needs. Young kids need help taking their enzymes and supplements, and the staff should understand that they need to be taken before all meals.

As kids with CF grow into teens, they'll probably begin eating more meals away from home and may feel pressured to eat in certain ways. Work with your teen, emphasizing the positive and monitoring any weight changes that are of concern. If you're packing lunch, include high-calorie options like ranch dressing and peanut butter crackers.

Increasing Caloric Intake

It can be tricky to make sure that kids with CF eat enough fat and calories, especially if they're sensitive to what's on the plates of siblings and peers. One way to increase calories without creating an entirely separate menu is to increase the calories and fat in one part of the meal.

Here are some simple ways to do so:

Add extra butter or margarine to sandwiches, sauces, and potatoes.

Use dressings on salads or vegetables; add extra oil to the dressing.

Prepare entrees with gravies and creamy sauces.

Add bacon to burgers and chicken.

Add dried skim milk powder to sauces and beverages.

Add extra cheese to scalloped potatoes or macaroni and cheese; order extra cheese on pizza.

Top salads and sandwiches with avocados or guacamole.

Add nuts to cookies, cakes, pancakes, and salads.

Add extra cheese and deli meats to sandwiches.

Grill sandwiches in butter or margarine.

Use heavy whipping cream and whole milk when cooking.

Make milkshakes.

Add instant breakfast mixes to milk-based beverages.

Make high-calorie smoothies.

Prepare calorie-rich desserts such as pudding and cheesecake.

Top hot chocolate, pudding, and other desserts with whipped cream.

Provide high-calorie snacks like peanut butter crackers or trail mix.

Prepare high-calorie versions of popular family recipes.

When you're shopping, be sure to compare the labels on similar prepared foods and purchase those that have higher fat and calorie content. Avoid diet foods — anything that claims to be nonfat, low fat, reduced calorie, or light/lite.

Look for whole-fat versions of dairy products such as sour cream, cottage cheese, and yogurt (many natural food stores carry a good variety of these). Don't forget to check baby food labels. You'd be surprised at how the caloric level varies between brands.

Beyond Food: Enzymes and Tube Feeding

Enzymes

A child with pancreatic insufficiency will need to take enzymes with meals and snacks to help digest food properly and to get the nutrition and fat needed to grow and gain weight.

Signs that your child may need enzymes or an enzyme dose adjustment include:

failure to gain weight, in spite of a strong appetite

frequent, large, greasy, or smelly bowel movements

bloating or gas

The CF dietitian or doctor will prescribe enzymes based on weight, growth, and how much a child eats at a time. An increased enzyme dose doesn't necessarily mean that a child is doing poorly. It may mean that he or she has gained weight and now requires more enzymes.

Enzymes need to be taken with every meal and most snacks. They come in capsules, full of tiny beads, that can be broken open for kids who are too young to swallow entire capsules. They should only be mixed with foods that are acidic, like applesauce. They should not be chewed or crushed. Never change the dose of enzymes without first consulting your child's dietitian or doctor.

Tube Feedings

In some cases, enzymes and high-calorie meals may not be enough to help a child thrive, and feedings through a tube into the stomach may be needed. It's not always physically possible for kids with CF to meet their increased energy needs by eating by mouth alone. Tube feedings, which can be done at night, are an excellent way to help kids who have trouble gaining weight, especially when they're ill.

Many kids and their parents are resistant to having a tube put in. But tube feedings are only prescribed when dietary interventions, calorie-boosting strategies, and enzymes haven't been enough. Tube feeding may sound scary at first, but tube insertion is usually an uncomplicated outpatient procedure. Results can include improved weight gain and decreased stress around eating.

Tube feedings do not mean that you've failed to feed your child well or that he or she isn't a good eater — often they just mean that a child needs so many calories a day that it's impossible to keep up without them. Your child's doctor and dietitian will make recommendations about tube feedings.

It can be hard for kids with CF to eat enough in a day to meet their needs, especially when they aren't feeling well. Although we all think we'd like to be able to eat a high-fat, high-calorie diet, many kids with CF, especially teens, find it difficult to do so in our diet-conscious culture.